Nursing Alsa

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Nursing Management in ALS

PHYSICA
L
I. Muscle Weakness/Skin Status

A. Assessment

1. Assess motor strength; presence of spasticity or flaccidity.
2. Assess for presence of contracture.
3. Assess skin daily, especially those areas susceptible to breakdown.

B. Common Nursing Diagnoses

1. Potential for injury related to impaired physical mobility.
2. Knowledge deficit regarding prevention of injury while promoting mobility
and self-care.
3. Alteration in comfort.

C. Management

1. Promotion of activity and exercise.

a) Encourage continuation of daily routines and activities.
b) Maintain exercise program to tone and strengthen unaffected muscle
groups without fatiguing affected muscles.
c) Range-of-Motion (ROM) exercised to prevent contracture and pain
in joints; first Active ROM, then Passive. Refer to physical therapist
for exercise program and see recommended passive range of motion
description in managing ALS (MALS) Manual II.
d) When weakness in the extremities begins to compromise mobility,
safety, or independence in ADL, refer to a physical or occupational
therapist.

The ALS Association, 27001 Agoura Road, Suite 150, Calabasas Hills, CA 91301-5104,
Phone: (800) 782-4747 / Fax: (818) 880-9006 / E-mail: alsinfo@alsa-national.org / Website: www.alsa.org

2. Promotion of proper positioning to prevent decubitus ulcers

a) Use as many different positions as possible when in bed. Change
positions every two hours, or on skin tolerance. After each change of
position, check for redness over bony prominences, and provide an
eggshell or circulating mattress when immobility prevents
independent repositioning.
b) Repositioning in the wheelchair based on the patient’s skin tolerance.
Use of a wheelchair cushion to prevent skin breakdown.
c) Proper positioning when ambulating or in a wheelchair, i.e., use of a
sling for a weak upper extremity.
d) Promote adequate nutritional intake.

II.
A. Assesment

1. Assess urination pattern and patterns of fluid intake.
2. Asses ability to transfer to toilet or commode, or standing ability for a
male.
3. Assess for signs and symptoms of urinary tract infection; frequency,
urgency, painful urination, fever, urinary tract infections are rare in ALS.


1. Impaired urinary elimination related to:

a) Progressive loss of mobility.
b) Dehydration.

C. Management

1. Encourage daily fluid intake of 2,5000cc’s per day unless contraindicated
because of swallowing ability.
2. Consult physician if there are signs of urinary tract infection.



III. Altered Bowel Function

A. Assessment

1. Assess bowel pattern (for constipation, diarrhea, impaction).
2. Assess diet, fluid intake, and swallowing ability.
3. Assess activity level.


1. Impaired bowel elimination.
2. Progressive loss of mobility
3. Progressive decline of dietary fiber.
4. Often mild/moderate dehydration.
5. Progressive inability of the trunk muscles to support a normal posture to
assist in defecation.

C. Management- Ideally focused on preventative measures. Unlike a neurogenic
bowel, person with ALS have slowed bowel motility.

1. Encourage patient to keep daily record of bowel movements (include
consistency and amount), particularly if there has been change to a more
constipated or less frequent stool.
2. Encourage daily fluid intake of 2,500cc’s per day unless contraindicated
because of swallowing ability.
3. provide dietary instruction of regarding importance of fiber, use of bran
cereals, prune juices, etc. unless contraindicated because of swallowing
ability.
4. Daily use of stool softeners and Metamucil are often recommended.
Laxatives may be used, but chronic use may be harsh on the bowel.
Mineral oil should not be used because of the danger of aspiration.
5. As mobility progresses and/or diet and fluid intake is altered, a more
aggressive bowel program may be indicated, daily or every other day.


NUTRITIO
N
I. Nutritional Needs
A. Assessment
1. Anthropometric Measures:
a) Height
b) Pre-morbid “usual” weight
c) Current Weight
d) Weight gain/loss pattern since onset of disease
e) “Ideal body weight”

2. Laboratory Test – indicated if patient has lost ten percent r more of body
weight in the last two months or weighs fifteen percent or more below
“ideal body weight.”
3. Hydration status – determined by careful recording of fluid intake and
output and by test of urine specific gravity. The average person needs
35cc fluid/kg body weight for adequate renal function. This figure can be
used to calculate fluid requirements.

B. Dysphagia (difficulty in swallowing)
1. Assessment

a) Assess gag, cough and swallowing reflexes, and chewing.
b) Assess patient’s ability to swallow liquids and solids.
c) Assess weight serially.

2. Common Nursing Diagnoses

a) Potential for injury.
b) Impaired nutritional status.
c) Knowledge deficit regarding alternatives for diet, food
preparation, and alternative procedures for supplemental feeding.
3. Management

a) Discuss techniques for protecting airway, i.e. sitting up straight,
putting chin on chest when swallowing, concentrating while
eating. Avoid increased stimulation i.e. talking at mealtime.
b) Refer to dietitian for determination of adequate nutritional intake
with continued weight loss.


C. Nutritional Management of the Dysphagic Patient

1. Promotion of optimum weight
a) Smaller, more frequent meals.
b) Foods that are “easy-to-eat”, i.e., adjusted mechanical soft diet.
c) Increased daily activity with non-fatiguing exercise.
d) Liquid supplement between meals.

RESPIRATOR
Y
A. Assessment

1. History: subjective symptoms (starving for air, shortness of breath and relation
to position changes, fatigue).
2. Assess: changes from baseline respiratory rate, depth, pattern, chest expansion;
adequacy of respiratory exchange and effort; retraction of intercostals spaces;
abdominal breathing; diaphragmatic breathing; nasal flaring and use of
cervical accessory muscles of respiration; color (cyanosis); cough, gag, and
swallow reflexes.
3. Auscultate: decreased breath sounds, presence of extra or adventitious sounds
(i.e., sales, wheezes).


1. Ineffective airway clearances related to:
a) Impaired/absent gag reflex.
b) Impaired/absent swallowing reflex.
c) Impaired/absent cough/sneeze reflex.

1. Impaired gas exchange related to:
a) Aspiration secondary to impaired/absent gag, swallowing and cough/
sneeze relexes.
b) Atelectasis secondary to impaired cough/sneeze reflexes and/or
hyperventilation.
c) Ineffective breathing pattern.

3. Knowledge deficit regarding:
a) Airway clearance and gas exchange.
b) Considerations regarding mechanical life supports.

C. Management

1. Maintain airway patency

a) Use of aspirator to suction secretions and prevent choking.
b) Use of medications (i.e., Elvail – 25 mg t.i.d. or 25-75 mg. at hs to
reduce secretions).
c) If the patient has a tracheostomy, suctioning and “trach care” should
be done prn to prevent buildup of secretions, possible obstruction
and infection.

2. Maintain adequate ventilation

a) Deep breathing and coughing exercises and use of incentive
spirometer to encourage lung expansion.
b) Chest physiotherapy: percussion, assisted cough and postural
drainage when indicated.
c) Oxygen, when ordered.
d) Elevation of the head of the bed with a foam wedge or hospital bed
to ease shortness of breath and provide comfort during sleep.
e) When air hunger is chronic and distressing, help monitor and titrate
medications that will help provide comfort.

3. Provide information and opportunities to discuss options for mechanical
ventilation. Generally, if there are early signs of dysphagia, dyspnea, or after
the patient has had time to adjust to his diagnosis, the patient and family
should be assisted in considering options regarding life support systems. The
important thing to remember is the patient should be thinking about these
decisions before he/she needs them.

4. Provide information and opportunity to discuss Physician’s Directive (also
called Living Will or Durable Power of Attorney for Medical Care). The pros
and cons of life support should be discussed in detail prior to the patient filling
out a Physician’s Directive.


Communication

A. Assessment
1. Assess volume and clarity of speech.
2. Assess ability to communicate needs to family/significant others.

1. Impaired verbal communications related to:
a) Altered volume of speech.
b) Altered clarity of speech (dysarthia).
c) Loss of speech.

C. Management
1. With dysarthria or with total loss of speech and no use of upper extremities.
There are a variety of alternative modes of communication.
2. Refer to a speech pathologist for evaluation and intervention.

Psychological Adaptation

A. Assessment
1. Evaluate the patient and family’s support systems and coping patterns with
awareness that with ongoing loss of independence, there will be ongoing
grieving by patient and family.

1. Ineffective coping strategies.
2. Knowledge deficit regarding alternatives for mobility and comfort.
3. Impaired support system.

C. Management
1. Provide an accepting environment in which the patient and family can share
concerns and fears with each other and members of the health care team.
2. Help the patient and family anticipate care needs and implications.
3. Anticipate and support emotional responses to loss as a normal reaction, thus
enabling the family to take in information and cope more effectively with
practical decision-making.
4. Provide encouragement and anticipate unasked questions.



5. Encourage realistic expectations of the disease whenever possible.
6. Refer, if needed, to a counselor, psychiatrist, psychologist or psychiatric
clinical nurse specialist.
7. Refer to an ALS support group if available.

—Excerpted from “Amyotrophic Lateral Sclerosis: A Teaching Manual for Health Professionals”, Nancy
Konikow, R.N., M.N., ALS Health Support Services, Kirkland, Washington.
—Rev. 6/96


Nursing Care Plan of
Amytrophic Lateral Sclerosis-Motor Neuron Disease

PROBLEMS/NEED GOALS/CRITERI NURSING
S A INTERVENTION
Weakness to flaccid paralysis Keep patient as active as -Active and passive ROM to
possible affected limbs 3-4x per day.
-Turn and position every 2
Prevent skin breakdown hours.-Keep skin dry.
-Wash skin and dry well after
each bowel movement or
urination (especially females).
-Lotion dry areas.-Air flotation
Prevent keep vein thrombosis, or egg crate mattress to bed.
pulmonary embolism -Elastic stockings, if ordered,
especially when patient out of
bed.

Emotional response to diagno- To assist patient through grief -Encourage verbalization of
sis and prognosis (anxiety, fear, and grieving process feelings.
denial, anger). -Accept behavior during stages
of grieving process and allow
grieving – teach family about
grieving process and encourage
them to accept patient’s behav-
ior and their own response.

To assist family to deal with -Use positive approach when
patient and grief process discussing progress (ask what
patient and family know about
disease).
-Encourage to maintain inde-
pendence for as long as pos-
sible and to do things for self
when possible – teach family to
do same.
-Plan diversionary activities,
OT, etc., while hospitalized –
have family plan same for at
home.


S A INTERVENTION
Altered body image and self- To assist patient in accepting -Discuss changes in body image
esteem new body image and maintain- and what they mean to patient.
ing self – esteem -Encourage patient to talk about
such with family.
-Encourage patient to focus on
positive aspects of self and to
share those aspects of self with
others.

(When bulbar symptoms occur) Maintain adequate 02 delivery -Suction and maintain patient
Respiratory insufficiency to patient; prevent complication airway
of pneumoniaAssisted cough -Administer 02 as necessary.
-Elevate HOB at least 30
degrees at all times.
-Auscultate chest frequently to
asses total airway states.
-Help patient to cough and deep
breathe at least every two hours
– may use incentive spirometer
if necessary or chest physical
therapy.
-Maintain on respirator if
necessary.

Difficulty swallowing and Prevent aspiration -Assess gag reflex before giving
chewing fluid or food by mouth.
Make food easier for patient to -Elevate HOB at mealtime and
eat for ½ hour afterward.
-Soft, mechanical soft or pureed
diet as needed.
Ensure adequate nutrition and -High protein, high CHO diet
fluid balance -Fluid intake 3000 cc/day.
-Consult dietary to teach basics
of good nutrition and in-be-
tween meal snacks.


S A INTERVENTION
Difficulty speaking Establish effective means of -Initiate use of a magic slate if
communication patient able to use hands.
-Initiate use of word boards or
letterboard if unable to use
arms.
-May use electro-larynx like
that used for laryngectomy
patients.
-Consult speech therapy and
OT for assistance and further
assessment of speech needs and
means for meeting these needs.

Urinary frequency or inconti- To maintain normal bowel and -Place urinal/bed pan where
nence of urine and feces of bladder function (usually do patient can reach if able to do
impaction remain normal until later stages so.
of disease) -Use effective means for patient
to communicate when needs to
urinate or have bowel move-
ment.
-Catheterize or when necessary
– external cath may be useful at
noc.
-Bowel routine as necessary for
impaction; high fiber diet if
patient can tolerate, Fleet
enema, Colace, Metamucil,
Signs/symptoms of illness, to notify M.D.: fluids, suppositories
Respiratory difficulty
Signs of infection – cold, flu, elevated temperature, chills
Difficulty swallowing
Difficulty speaking
Sudden increase in original signs and symptoms of disease

Rev. 10/96. This material is the property of The ALS Association and may not be edited or excerpted. To obtain original
reprints, contact The ALS Association.


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